Esophageal stenting in children: indications, application, effectiveness, and complications.

BACKGROUND: Use of esophageal stents is uncommon in children, and there are few reports. We report the first experience in predominantly small children and infants with retrievable, flexible stents designed for tracheobronchial use. OBJECTIVE: Evaluation of initial experience with placement of esophageal stents for benign esophageal disorders in children. DESIGN: A retrospective study. SETTING: A pediatric, academic, tertiary-referral center. PATIENTS: This study involved 7 pediatric patients. INTERVENTIONS: Covered tracheobronchial stents were endoscopically placed in pediatric patients with benign esophageal conditions. Removal involved using forceps to pull the purse-string suture into the endoscope channel and collapsing the top of the stent for easy removal. MAIN OUTCOME MEASUREMENTS: To evaluate the safety and feasibility of performing endoscopic stent placement in children and to establish criteria for early stent removal. RESULTS: Six of 7 patients benefitted from stenting. There were no complications of placement. Novel techniques were developed for difficult retrievals. One patient did not benefit from esophageal stent placement, because the stent migrated downward from the uppermost part of the esophagus. One patient had some gagging, which led to early removal of the stent. A stent was removed emergently in 1 patient for respiratory distress. LIMITATION: Small number of patients. CONCLUSIONS: Retrievable, covered stents are easily placed and removed from the esophagus in small children. They should be considered for severe unrelenting strictures, especially when associated with esophageal leaks. A need exists for development of esophageal stents designed for pediatric use.

Long-gap esophageal atresia treated by growth induction: the biological potential and early follow-up results.

This study had two purposes. The first was to determine whether the growth procedure would allow true primary repairs of the most severe end of the esophageal atresia (EA) spectrum with the longest gaps (LG) and most rudimentary lower esophageal segments. The second goal was to provide the first short- to mid-term (3-12 years) follow-up data on the esophageal function and quality of life (QOL) data on the patients in this series. From our series of 60 LG-EA patients who underwent a growth procedure, 42 had the true primary esophageal repair completed 3 years ago. Among these, 18 had gaps over 6 cm, and for 6, only a rudimentary lower esophagus existed well below the diaphragm. No patient was turned down and all had primary repairs. These results suggest that even the most rudimentary segment has the potential to achieve normal size and that the full EA spectrum can have a primary repair. Our follow-up studies indicated that the esophageal function of these previously grown segments was very good. All contacted (40) were eating normally with only 3 receiving supplemental g-tube feeds because of other significant defects. We have actively treated significant reflux and 41/42 had fundoplication. By endoscopy (N = 15) no esophagitis was visible, but on biopsy, mild inflammation was found in 3. No conditions were found which would suggest that there would be a late deterioration or adverse consequences would arise. Based on these ongoing evaluations, the outlook seems very favorable for a good long-term QOL.

Categorization and repair of recurrent and acquired tracheoesophageal fistulae occurring after esophageal atresia repair.

PURPOSE: Recurrent trachea-esophageal fistula (recTEF) is a frequent (5%-10%) complication of congenital TEF (conTEF) and esophageal atresia (EA) repair. In addition, postoperative acquired TEF (acqTEF) can occur in addition to or even in the absence of prior conTEF in the setting of esophageal anastomotic complications. Reliable repair often proves difficult by endoluminal or standard surgical techniques. We present the results of an approach that reliably identifies the TEF and facilitates airway closure as well as repair of associated tracheal and esophageal problems. METHODS: Retrospective review of 66 consecutive patients 2009-2016 (55 referrals and 11 local) who underwent repair via reoperative thoracotomy or cervicotomy for recTEF and acqTEF (IRB P00004344). Our surgical approach used complete separation of the airway and esophagus, which reliably revealed the TEF (without need for cannulation) and freed the tissues for primary closure of the trachea and frequently resection of the tracheal diverticulum. For associated esophageal strictures, stricturoplasty or resection was performed. Separation of the suture lines by rotational pexy of the both esophagus and the trachea, and/or tissue interposition were used to further inhibit re-recurrence. For associated severe tracheomalacia, posterior tracheopexy to the anterior spinal ligament was utilized. RESULTS: The TEFs were recurrent (77%), acquired from esophageal leaks (26%), in addition to persistent or missed H-type (6%). Seven patients in this series had multiple TEFs of more than one category. Of the acqTEF cases, 6 were esophagobronchial, 10 esophagopulmonic, 2 esophagotracheal (initial pure EA cases), and 2 from a gastric conduit to the trachea. Upon referral, 18 patients had failed endoluminal treatments; and open operations for recTEF had failed in 18 patients. Significant pulmonary symptoms were present in all. During repairs, 58% were found to have a large tracheal diverticulum, and 51% had posterior tracheopexy for significant tracheomalacia. For larger esophageal defects, 32% were treated by stricturoplasty and 37% by segmental resection. Rotational pexy of the trachea and/or esophagus was utilized in 62% of cases to achieve optimal suture line separation. Review with a mean follow-up of 35months identified no recurrences, and resolution of pulmonary symptoms in all. Stricture treatment required postoperative dilations in 30, and esophageal replacement in 6 for long strictures. There was one death. CONCLUSION: This retrospective review of 66 patients with postoperative recurrent and acquired TEF following esophageal atresia repair is the largest such series to date and provides a new categorization for postoperative TEF that helps clarify the diagnostic and therapeutic challenges for management.

Jejunal Interposition after Failed Esophageal Atresia Repair.

BACKGROUND: The early outcomes of using jejunal interpositions to establish esophageal continuity in patients who have had a failed repair of esophageal atresia (EA) were determined. STUDY DESIGN: This was a retrospective review of all patients treated at our institution with a jejunal interposition after a failed EA repair from 2010 to 2015. Demographics, anatomy encountered, operative techniques, requirement for microvascular support, and length of stay were analyzed. Outcomes measures included conduit survival, as well as feeding status at last follow-up. RESULTS: Ten patients were reviewed. Median age at time of interposition operation was 48 months (range 8 to 276 months) and median weight was 14.2 kg (range 7.2 to 49.7 kg). Preoperative anatomy, operative techniques, and outcomes are presented. Four patients had microvascular "supercharging" for a long jejunal graft. Median follow-up was 1.5 years (range 0.5 to 5 years) with no long-term loss of graft or deaths. Six patients are eating by mouth completely, 1 by mouth primarily with supplemental night-time feeds, 1 is transitioning from tube to oral feeds, and 2 with functional grafts are fed mostly enterally due to severe oral aversion in 1 and aspiration in 1. CONCLUSIONS: Jejunal interpositions have been used for the past 5 years to establish esophageal continuity after a failed EA repair. All jejunal conduits survived and were joined to the upper esophageal segment. For shorter gaps with a longer upper esophageal pouch, a thoracic esophageal anastomosis was possible without additional vascular support. For longer interpositions into the neck, upper conduit survival might benefit from additional vascular anastomoses (ie, supercharging). To provide adequate space in the mediastinum, the first rib can be removed, as well as a portion of the manubrium to enlarge the pathway into the neck.

A flexible approach to achieve a true primary repair for all infants with esophageal atresia.

Our purpose is to present our results using a flexible surgical approach to achieve a true primary repair for all infants with esophageal atresia (EA). The proposed methods are designed to reach this goal, even when most of the intrathoracic esophagus is missing. What has made this goal attainable is the ability to rapidly induce esophageal growth. We reviewed the results of 63 consecutive patients who presented between 1984 and 2004 with an esophageal gap of greater than 2.5 cm, a distance where increasingly difficult repairs begin. Gaps of 4 cm or longer (46), 6.0 cm or longer (14), and over 10 cm (3) were included. Repairs begun elsewhere (20) included 17 spit fistulas. We present 3 surgical stages, designed to overcome tension and, for longer gaps, induce esophageal growth. For 25 infants, traction in the operating room was sufficient to dissipate tension and allow a primary repair. For the longest gaps, successful esophageal growth was induced over days in 38 patients by internal (5), external (23), and mixed internal/external traction (10). Growth quickly produced adequate esophagus for a primary repair. We conclude that adequate esophageal growth can be induced within days, even early in infancy, and this flexible approach allows the entire EA spectrum to be repaired primarily.

Foker process for the correction of long gap esophageal atresia: Primary treatment versus secondary treatment after prior esophageal surgery.

PURPOSE: The Foker process (FP) uses tension-induced growth for primary esophageal reconstruction in patients with long gap esophageal atresia (LGEA). It has been less well described in LGEA patients who have undergone prior esophageal reconstruction attempts. METHODS: All cases of LGEA treated at our institution from January 2005 to April 2014 were retrospectively reviewed. Patients who initially had esophageal surgery elsewhere were considered secondary FP cases. Demographics, esophageal evaluations, and complications were collected. Median time to esophageal anastomosis and full oral nutrition was estimated using the Kaplan-Meier method. Multivariate Cox-proportional hazards regression identified potential risk factors. RESULTS: Fifty-two patients were identified, including 27 primary versus 25 secondary FP patients. Median time to anastomosis was 14 days for primary and 35 days for secondary cases (p<0.001). Secondary cases (p=0.013) and number of thoracotomies (p<0.001) were identified as significant predictors for achieving anastomosis and the development of a leak. Predictors of progression to full oral feeding were primary FP cases (O.R.=17.0, 95% CI: 2.8-102, p<0.001) and patients with longer follow-up (O.R.=1.06/month, 95% CI: 1.01-1.11, p=0.005). CONCLUSIONS: The FP has been successful in repairing infants with primary LGEA, but the secondary LGEA patients proved to be more challenging to achieve a primary esophageal anastomosis. Early referral to a multidisciplinary esophageal center and a flexible approach to establish continuity in secondary patients is recommended. Given their complexity, larger studies are needed to evaluate long-term outcomes and discern optimal strategies for reconstruction.

Direct tracheobronchopexy to correct airway collapse due to severe tracheobronchomalacia: Short-term outcomes in a series of 20 patients.

PURPOSE: Tracheobronchomalacia (TBM) is associated with esophageal atresia, tracheoesophageal fistulas, and congenital heart disease. TBM results in chronic cough, poor mucous clearance, and recurrent pneumonias. Apparent life-threatening events or recurrent pneumonias may require surgery. TBM is commonly treated with an aortopexy, which indirectly elevates trachea's anterior wall. However, malformed tracheal cartilage and posterior tracheal membrane intrusion may limit its effectiveness. This study describes patient outcomes undergoing direct tracheobronchopexy for TBM. METHODS: The records of patients that underwent direct tracheobronchopexy at our institution from January 2011 to April 2014 were retrospectively reviewed. Primary outcomes included TBM recurrence and resolution of the primary symptoms. Data were analyzed by McNemar's test for matched binary pairs and logistic regression modeling to account for the endoscopic presence of luminal narrowing over multiple time points per patient. RESULTS: Twenty patients were identified. Preoperative evaluation guided the type of tracheobronchopexy. 30% had isolated anterior and 50% isolated posterior tracheobronchopexies, while 20% had both. Follow-up was 5 months (range, 0.5-38). No patients had postoperative ALTEs, and pneumonias were significantly decreased (p=0.0005). Fewer patients had tracheobronchial collapse at postoperative endoscopic exam in these anatomical regions: middle trachea (p=0.01), lower trachea (p<0.001), and right bronchus (p=0.04). CONCLUSION: The use of direct tracheobronchopexy resulted in ALTE resolution and reduction of recurrent pneumonias in our patients. TBM was also reduced in the middle and lower trachea and right mainstem bronchus. Given the heterogeneity of our population, further studies are needed to ascertain longer-term outcomes and a grading scale for TBM severity.

Surgical approaches to aortopexy for severe tracheomalacia.

PURPOSE: The purpose of this study was to determine the outcomes among three different surgical approaches for performing an aortopexy to treat severe tracheomalacia (STM). METHODS: A retrospective review was performed for all patients who underwent an aortopexy by pediatric surgeons at a single institution during 1997-2012. Data collected included details of the operative approaches and clinical results. The data were analyzed using Chi-square and Fisher exact test. RESULTS: Forty-one patients underwent an aortopexy. The operation was chosen by the surgeon and not randomized. Exposure was by partial sternotomy (PS) (20), open thoracotomy (12), or thoracoscopic approach (7). Only the PS approach was done by a single team. All groups showed improvement in work of breathing, prevention of severe respiratory distress, and acute life threatening events. These effects were more dramatic for the PS group, especially regarding oxygen and/or ventilator dependence and the ability to undergo tracheostomy decannulation. Among the sixteen patients with failure-to-thrive before successful aortopexy by any technique, ten demonstrated significant improvement in their growth (p=0.025). The recurrence rate for the thoracoscopic approach was 38%, and there were no recurrences in the partial sternotomy and the thoracotomy groups, 38% vs 0% vs 0%, p=0.005. Simultaneous bronchoscopy was utilized more commonly in the PS group compared to the thoracotomy and thoracoscopic group, 95% vs 62% vs 38%. CONCLUSIONS: In this series, the partial sternotomy technique had the most reliable resolution of symptoms and no recurrence requiring reoperation. The PS approach to STM has the technical advantages of an improved exposure with equal access to the vessels over the right and left mainstem bronchi, as well as the trachea and a more specific elevation of the arteries, including suspension of the pulmonary arteries and trachea itself when desirable. Simultaneous bronchoscopy during aortopexy and an experienced team also likely contribute to improved outcomes. The variations in populations, follow-up, and use of continuous intraoperative bronchoscopy, however, make firm conclusions difficult.

Two-ventricle repairs in the unbalanced atrioventricular canal defect spectrum with midterm follow-up.

OBJECTIVES: Unbalanced atrioventricular (AV) canal defects include a hypoplastic ventricle (HV) and AV valve (HAVV) precluding complete 2-ventricle repairs (2VRs). Catch-up growth would solve this problem and was induced by increasing HAVV flow. The objectives were to assess reliability of HV and HAVV growth and provide 5- to 15-year 2VR follow-up. METHODS: From 1990 to 2005, 23 consecutive infants (13 females and 10 males) with echo-diagnosed unbalanced AV canal defects (n = 20) or subsets (n = 3) underwent 2VRs. HV volumes (18 left and 5 right) and HAVV sizes estimated from biplane echoes and z values (standard deviation from expected) were determined. Hypoplasia was defined by a z value of less than -2.0. Three operative approaches were used: (1) Staged repairs (n = 9) had complete AVV repairs with partial atrial septal defect and ventricular septal defect closures, which increased HAVV flow and maintained stability. The septal defects were closed later. (2) An asymmetric valve partition (n = 8) was used to increase HAVV size. (3) For moderate hypoplasia, HAVV flow was increased and ASDs/VSDs were left for stability (n = 6). Follow-up at 5 to 19 years was done locally. RESULTS: Staged repairs began at 20 to 328 days (average, 129 days) and were completed 5 to 145 days later (average, 101 days). Midterm survival was 87% (20/23) after 1 central nervous system bleed after trial weaning from extracorporeal membrane oxygenation and 2 later deaths from hyperkalemia. Reoperations for AVV regurgitation (n = 3), AVV stenosis (n = 1), and mitral valve replacement (n = 1) were satisfactory. On follow-up, all hypoplastic structures (HV and HAVV) had grown to normal size. Two patients "doing well" were lost to follow-up. Survivors have satisfactory 2VRs, with 15 of 18 taking no cardiac failure medications. CONCLUSIONS: Reliable HV/HAVV catch-up growth was induced, and all midterm 2VRs were satisfactory.

The Foker technique (FT) and Kimura advancement (KA) for the treatment of children with long-gap esophageal atresia (LGEA): lessons learned at two European centers.

INTRODUCTION: We present the experiences from two European centers performing the Foker technique (FT) of esophageal lengthening by axial traction and the Kimura advancement (KA) method of lengthening the upper pouch by extrathoracic resiting a spit fistula (SF) in children with long-gap esophageal atresia (LGEA, gap length > 5 cm). MATERIALS AND METHODS: A total of 15 children were treated (8 pure EA, 6 lower tracheoesophageal fistula [TEF], and 1 upper TEF). Gaps ranged from 5 to 14 cm. Nine children already had a SF. Patients were grouped according to the presence of a SF and the subsequent surgical strategy: Group A (no SF, n = 6) received FT on both pouches. Group B (with SF, n = 6) received KA of SF and FT of the lower pouch. Group C (with SF, n = 3) received closure of the SF and subsequent Foker traction (CSFT) on both pouches. RESULTS: Group A: Primary repairs for all six children (mean age 3 months, gap length 6.5 cm) after a mean traction time of 3 weeks and a mean of 2.1 thoracotomies (range 2 to 3). Dilations were required in three out of six for anastomotic strictures with one perforation during the second dilation. Group B: All six children (mean age 16.4 months, gap length 9.5 cm) had a primary anastomosis, although for two it was significantly delayed (48 and 143 weeks traction time) because of infections. The number of thoracotomies ranged from 2 to 8 (mean 3.6). Leaks occurred in five out of six anastomoses (responsive to conservative management). Two children developed severe strictures, which required the anastomosis to be redone. In group C (mean age 10.6 months, gap length 6.5 cm), several major complications occurred. The three SF closures leaked (one iatrogenic) causing severe mediastinitis. CSFT was successful in only one case and the other two children had an esophageal replacement (stomach, jejunum). No deaths occurred in the series. CONCLUSION: FT of both pouches (group A) resulted in primary repairs of all six LGEA patients. The combination of KA and FT (group B) resulted in an equivalent rate of primary repairs, but with an increased number of thoracotomies and rate of complications compared with group A. CSFT (group C) resulted in a high failure rate. More data are needed (we propose a multicenter registry) to elucidate the safety and efficacy of each elongation technique and to establish an algorithm with clearer inclusion and exclusion criteria.

Surgical advances in the fetus and neonate: esophageal atresia.

This article focuses on selected topics in the diagnosis and management of patients with esophageal atresia (EA) with or without tracheoesophageal fistula. The current status of prenatal diagnosis and recent advances in surgical techniques, including thoracoscopic repair for short-gap EA and tension-induced esophageal growth for long-gap EA, are reviewed. Although no consensus exists among pediatric surgeons regarding the role of these procedures in the treatment of EA, one can reasonably expect that, as they evolve, their application will become more widespread in this challenging patient population.

Mitral and tricuspid valve repair and growth in unbalanced atrial ventricular canal defects.

OBJECTIVE: Congenital mitral and tricuspid valve abnormalities in unbalanced atrioventricular canal defects are complex. We designed procedures to both repair and induce growth of hypoplastic atrioventricular valves and ventricles to achieve 2-ventricle repairs. Midterm data were assessed for reliability of catch-up growth, resulting quality of atrioventricular valves, and adequacy of 2-ventricle repairs. METHODS: The 24 consecutive infants (14 female and 10 male) with unbalanced atrioventricular canal defects had significant hypoplasia of 1 atrioventricular valve and/or ventricle (an echocardiography-derived z value of ≤-3.0 standard errors of the mean below expected). Operative approaches included the following: (1) Staged repair was performed, with complete valve repair, partial closure of the atrial septal, and ventricular septal defects, and (usually) pulmonary artery banding. After adequate growth, repair was completed. A vestigial mitral valve (4-7 mm) in 3 patients led to partitioning the large tricuspid valve, creating a second mitral valve. (2) Repair with a shift in atrioventricular valve partitioning was performed to increase hypoplastic atrioventricular valve size. (3) Repair with snared atrial septal defects and ventricular septal defect was performed to allow intracardiac shunting. The hypoplastic atrioventricular valves and hypoplastic ventricles were reassessed on local follow-up (5-15 years). RESULTS: The initial z scores were -2.8 to -7.4 for hypoplastic atrioventricular valves and -1.0 to -7.5 for hypoplastic ventricles. Follow-up z scores were -0.6 to -2.7 for hypoplastic atrioventricular valves and -2.0 to +1.8 for hypoplastic ventricles. Another 11 patients were also judged to be within normal limits. Three reoperations were for mitral valve regurgitation, and 1 reoperation was for mitral valve replacement. One patient died of central nervous system bleed just before extracorporeal membrane oxygenation weaning, and 2 patients died of late potassium overdose, for an 88% survival. Survivors are well with 2-ventricle repairs, and 15 of 19 patients are not taking cardiac medications. CONCLUSIONS: Increasing atrioventricular valve flow reliably induced growth. Valve repair and growth achieved a 2-ventricle repair in all patients.

Achievement of feeding milestones after primary repair of long-gap esophageal atresia.

OBJECTIVES: To determine the pattern of feeding milestones following primary repair of long-gap esophageal atresia (EA). METHOD: A questionnaire based upon well established feeding milestones was used. Children after long-gap EA repair, n=40, were compared from after primary repair to healthy children from birth, n=102. RESULTS: The age when surveyed of the EA group and controls was different: 6.2+/-4.7 (mean+/-standard deviation) years, range 1.1-20.9, versus 2.5+/-2.4 years, range 0.0-12.1, p=0.00. The esophageal gap length in the EA group was 5.1+/-1.2 cm and age at repair was 5.5+/-5.0 months. There was no statistically significant difference between the atresia group and controls for feeding milestones; Self feeding finger foods approached significance. There was, however, greater variability in the timing of milestones in the atresia group compared to controls. Feeding milestones were negatively correlated with age at primary repair: drinking with a covered sippy cup, rho=-0.51, p=0.01 and self feeding finger foods, rho=-0.36, p=0.04 were statistically significant. Drinking from a cup correlated with gestational age, rho=0.38, p=0.04, and negatively correlated to esophageal gap length, rho=-0.45, p=0.01. CONCLUSIONS: Despite delayed onset of feeding, major milestones after EA repair occurred in similar pattern to normal infants. An early referral for primary repair is beneficial for earlier acquisition of milestones for infants with long-gap EA.

Combination of spit fistula advancement and external traction for primary repair of long-gap esophageal atresia.

Primary repair of long-gap esophageal atresia with almost complete absence of thoracic esophagus was usually believed to be impossible. Thus, esophageal replacement with colon or gastric interposition seemed inevitable. Esophageal lengthening techniques could be an alternative approach. Herewith we describe for the first time the successful combination of the stepwise subcutaneous advancement of the upper esophageal segment (Kimura's technique) with transthoracic traction on the lower esophageal segment (Foker's technique). This combined lengthening technique leads to the primary repair of a long-gap esophageal atresia.

Staged esophageal lengthening with internal and subsequent external traction sutures leads to primary repair of an ultralong gap esophageal atresia with upper pouch tracheoesophagel fistula.

Primary repair of very long gap esophageal atresia (EA) with almost complete absence of thoracic esophagus has usually been thought impossible. Thus, esophageal replacement with colon or gastric interposition seemed inevitable. In contrast, J. Foker described a technique of lengthening the pouches with traction sutures and making primary repair possible. To contribute clinical experience to this discussion, we report about esophageal elongation in a child with long gap EA and an upper pouch tracheoesophageal fistula (TEF). The patient presented as a preterm baby with a long gap EA of almost 9 vertebral bodies (7 cm) and additionally TEF on the upper pouch. Initially, he was treated with a gastrostomy and replogle suction of the upper pouch. Tracheoesophageal fistula was repaired, and the upper pouch brought from the neck into the thoracic inlet. At the same time thoracotomy was performed, and the lower esophageal segment mobilized and fixed to the prevertebral fascia under moderate tension. The tension reduced the gap between both pouches to about 3.5 cm. After 4 weeks, both pouches were mobilized further. However, the remaining gap did not allow primary anastomosis at that time, so the traction sutures were reconfigured and brought out externally through the skin above and below the incision. Daily increases in tension resulted in the ends virtually touching within 10 days. Now a contrast study showed the two lumens within 5 mm of each other, and primary anastomosis was completed without difficulty. Postoperative diagnosed gastroesophageal reflux and anastomotic stricture were controlled by a Thal hemifundoplication and dilatations. In conclusion, staged esophageal lengthening may be considered for a primary repair of EA even in cases with ultralong gap and TEF.

Treatment of right ventricle to coronary artery connections in infants with pulmonary atresia and intact ventricular septum.

OBJECTIVE: At the severe end of the spectrum of infants with pulmonary atresia and intact ventricular septum, the likelihood of significant right ventricle to coronary artery connections increases. Our purpose is to present the first series of right ventricle to coronary artery connections ligated off bypass before right ventricular decompression and to evaluate the consequences of this approach. METHODS: From 1988 to 2007, 19 patients with pulmonary atresia and intact ventricular septum had a total of 69 right ventricle to coronary artery connections identified preoperatively, and 10 more were located intraoperatively. Of these, 71 were judged large enough to warrant off-pump direct ligation. Preoperative diagnosis was by transthoracic echocardiography and angiography. Transesophageal and surface echocardiography were used for intraoperative location. Direct visualization and echocardiographic assessment for regional wall motion abnormalities determined the effects of ligation. Right ventricular decompression was done in all patients. RESULTS: After ligation, coronary flow converted from moderately or largely retrograde to antegrade perfusion. Ligation produced no visual myocardial consequences or immediate local wall motion abnormalities. For 3 patients, however, apical-septal wall motion abnormalities appeared from 2 hours to 3 days postoperatively. Serial studies were done to assess the later effects in the 16 of 19 30-day survivors. No evidence for myocardial injury was found, and all continued on a 2-ventricle repair course. CONCLUSION: The location and ligation of right ventricle to coronary artery connections can be reliably accomplished off bypass. Coronary flow became antegrade, improving myocardial oxygenation. No myocardial damage was observed. Inapparent right ventricle to coronary artery connections occasionally enlarge secondarily after right ventricular decompression, making early follow-up evaluation necessary after ligation. Despite the initial presence of significant right ventricle to coronary artery connections, 2-ventricle repairs are possible with long-term benefits.

Use of high-resolution endoscopic ultrasonography to examine the effect of tension on the esophagus during primary repair of long-gap esophageal atresia.

BACKGROUND: We have shown that tension applied to the esophageal pouches in long-gap esophageal atresia allows primary repair without necessity for intestinal or gastric transposition. OBJECTIVE: To determine whether the mural structure of the upper esophageal pouch is altered by tension. MATERIALS AND METHODS: We examined the esophagus with high-resolution endoscopic ultrasonography. The upper pouch was examined before traction and the upper and lower esophagus were examined after primary repair. Of 11 patients examined, 7 were male. At initial surgery the age, weight and length of the patients (mean +/- SD) were 118 +/- 88 days, 4.7 +/- 1.2 kg and 54 +/- 4 cm, respectively. The gap length was 4.7 +/- 1.1 cm. RESULTS: The pretraction measurement of the muscularis propria of the upper pouch was similar to the postanastomotic measurement of the upper esophagus, and there was no statistically significant difference from the lower esophageal segments after anastomosis: 0.83 +/- 0.19, 0.80 +/- 0.15 and 0.81 +/- 0.22 mm, respectively (P = 0.90). The thickness of combined mucosa and submucosa was also very similar in all three measurements, respectively: 0.93 (0.21) mm vs. 1.06 (0.08) mm vs. 1.0 (0.11) mm (P = 0.14). CONCLUSION: The layers of the upper esophageal pouch are preserved in infants with esophageal atresia in whom esophageal length is increased with tension.

Postoperative coagulopathy in a pediatric patient after exposure to bovine topical thrombin.

Severe postoperative coagulopathy developed in a child with congenital heart disease due to a factor V inhibitor from repetitive exposure to bovine topical thrombin. This case report alerts pediatric providers to consider these inhibitors when postoperative coagulopathy occurs. Potential treatment options are reviewed.

The effect of traction on esophageal structure in children with long-gap esophageal atresia.

We examined the esophageal structure in children who underwent traction to achieve primary repair of long-gap esophageal atresia. High-resolution ultrasound was used to compare thickness of the proximal and distal esophagus in children who had traction to achieve primary repair (n=15) to cases of esophageal atresia with shorter gaps that did not require traction (n=8). The muscularis propria of the upper esophagus was thicker in the traction compared to the non-traction group, though not statistically significant (respectively, 0.79 (0.18) mm vs. 0.71 (0.16) mm; p=0.29), measurements were similar for the lower esophagus (respectively, 0.79 (0.21) mm vs. 0.75 (0.13) mm; p=0.64). Combined mucosa and submucosa was very similar in both groups for the upper (respectively, 1.03 (0.15) mm vs. 1.04 (0.16) mm; p=0.95) and lower esophagus (respectively, 1.09 (0.23) mm vs. 1.01 (0.13) mm; p=0.37). The thickness of individual mural layers is maintained after increasing esophageal length with traction.